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Year : 2019  |  Volume : 22  |  Issue : 5  |  Page : 734-738

Idiopathic acute massive pulmonary embolism in childhood

1 Department of Emergency Medicine, Medical Faculty, Ahi Evran University, Kirsehir, Turkey
2 Department of Cardiyology, Medical Faculty, Ahi Evran University, Kirsehir, Turkey
3 Department of Chest Medicine, Medical Faculty, Ahi Evran University, Kirsehir, Turkey

Date of Acceptance26-Nov-2018
Date of Web Publication15-May-2019

Correspondence Address:
Dr. B Celik
Güldiken Mahallesi, Şehit Polis Ekrem Ülker Caddesi, No: 18-1/10, Merkez, Kırşehir
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/njcp.njcp_263_18

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Acute pulmonary embolism (PE) is an uncommon clinical condition in childhood. We hereby present a case report of a 10-year-old child who presented to the emergency department with an acute massive PE. He was transferred by ambulance to our emergency department for dyspnea and perioral cyanosis. His parents denied any previous history of illness or familial disease. Arterial blood gas analysis, electrocardiography, and clinical symptoms and signs collectively raised a suspicion of a probable PE. A contrast-enhanced pulmonary computed tomography scan revealed a massive thrombus in the distal part of the right pulmonary artery with no contrast passage into upper, middle, and lower lobar arteries. Upon ascertaining, the diagnosis of PE, intravenous saline infusion, 3 L/min oxygen through nasal route, and subcutaneous enoxaparin 0.4 cc was administered promptly. As our hospital lacked a pediatric intensive care unit and a further need for administration of pharmacological thrombolysis was anticipated, we transferred the patient to a tertiary care center. PE should always be kept in mind as a differential diagnosis in emergency departments even in pediatric patients.

Keywords: Childhood, idiopathic, pulmonary embolism

How to cite this article:
Caliskan H M, Ozbek S C, Celik B, Sokmen E, Karayigit D Z, Savas O. Idiopathic acute massive pulmonary embolism in childhood. Niger J Clin Pract 2019;22:734-8

How to cite this URL:
Caliskan H M, Ozbek S C, Celik B, Sokmen E, Karayigit D Z, Savas O. Idiopathic acute massive pulmonary embolism in childhood. Niger J Clin Pract [serial online] 2019 [cited 2019 Aug 24];22:734-8. Available from:

   Introduction Top

Acute pulmonary embolism (PE) is an uncommon clinical condition in childhood that requires a timely diagnosis and management due to its potentially fatal nature. Its rarity among children compared to the adult population together with its high possibility of being overlooked by physicians highlights PE as a disease which comes as the last differential diagnosis to the physicians' consideration, especially among the pediatric population. Since the first time, a case of pediatric PE was reported in 1861,[1] little progress has been achieved in the derivation of clinical decision rules for childhood PE due to its much lower incidence in children compared to adults, confusing presenting complaints, and different risk factors. We hereby present a case report of a 10-year-old nonobese child with no other known traditional risk factors, who presented to the emergency department with the acute massive PE.

Written informed consent was obtained from the parents of the patient. Local ethics committee approval was not required for this paper due to the fact that the paper was a case report.

   Case Report Top

A 10-year-old boy was referred by ambulance to our emergency department from a referring countryside hospital for dyspnea and perioral cyanosis, along with an intact 22-G intravenous line, 2 L/min nasal oxygen support, and telemetric and oximetric motorization. His parents denied any previous history of illness or familial disease but reported that he had been complaining about occasional chest pain for the last 3 days. The initial physical examination revealed the following findings: dyspnea with respirations 28/min, tachycardia with a heart rate of 128 beats/min, mild confusion, severe hypotension in the supine position with blood pressure 60/30 mmHg, and hypoxia with pulse oximeter reading 88% in room air. According to the findings, there could be different options in terms of diagnosis such as severe asthma attack or pneumonia and congenital heart diseases. On the other hand, the patient's cardiac and pulmonary auscultations findings did not support any of the aforementioned diagnoses. Arterial blood gas analysis was compatible with hypoxia, hypocapnia, and respiratory alkalosis (pH 7.49, PO229.2 mmHg, SO288%, and pCO229.3 mmHg). Electrocardiography (ECG) depicted sinus tachycardia, incomplete right bundle block, mild T-wave negativity in chest leads, and S1Q3T3 pattern [Figure 1]. Moreover, these clinical symptoms and signs collectively raised a suspicion for a probable PE and urged us to ask for a cardiology consultation for echocardiographic evaluation. Echocardiography revealed normal left ventricular systolic functions and dilatation in the inferior vena cava, right atrium, and ventricle [Figure 2], along with the moderate degree of tricuspid regurgitation and a predicted pulmonary systolic pressure of around 50 mmHg. Accordingly, we considered implementing a contrast-enhanced pulmonary computed tomography (CT) scan, which revealed a massive thrombus in the distal part of the right pulmonary artery with no contrast passage into upper, middle, and lower lobar arteries [Figure 3].
Figure 1: Electrocardiography on admission

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Figure 2: Echocardiographic view

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Figure 3: Computed tomographic angiography

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Blood chemistry, hemogram, and troponin I level were observed to be normal. Serum D-dimer level was 1.27 mg/L (normal <0.55 mg/L). Doppler ultrasound of the lower extremities did not reveal any thrombus formation in the legs. Upon ascertaining, the diagnosis of PE, intravenous saline infusion, 3 L/min oxygen through nasal route, and subcutaneous enoxaparin 1 mg/kg (0.4 cc) was administered promptly. As our hospital lacked any pediatric intensive care unit and a need for administration of thrombolytic treatment was anticipated, we transferred the patient by ambulance to a tertiary care center.

In the tertiary center, the patient received 0.3 mg/kg/h (24 mg total) alteplase for 2 h as thrombolytic therapy and unfractionated heparin infusion 20 U/kg/h for 24 h for acute cor pulmonale and severe hypotension. Furthermore, intravenous pantoprazole 1 mg/kg (40 mg total) once daily was added for gastric protection. Lower and upper extremity Doppler ultrasound imaging in the tertiary center also did not show any thrombus formation. After thrombolytic therapy, the patient's general status improved, however, echocardiographic reexamination revealed the persistence of the right atrial and ventricular dilatation, moderate degree tricuspid regurgitation, and pulmonary hypertension.

Further laboratory tests to inquire thrombophilia revealed normal Factor V Leiden and prothrombin 20210 genes, along with heterozygous mutations in methylenetetrahydrofolate reductase (MTHFR) C677T, MTHFR A1298C, and plasminogen activator inhibitor-1 (PAI-1): 4G/5G. Plasma homocysteine level was 12.1 μgr/L (normal <15 μgr/L). The results of coagulation tests were as follows: antithrombin III activity, 80.9% (normal: 75%–125%); protein C activity, 68.83% (normal: 70%–140%); protein S activity, 70.14% (normal: 60%–130%); factor VIII activity, 151.66% (normal: 70%–150%); and Lupus A ratio, 1.14% (negative result). Moreover, serologic tests for anticardiolipin immunoglobulin M (IgM) and IgG showed negative results.

The patient was prescribed enoxaparin 1 mg/kg twice daily and lansoprazole 30 mg once daily and discharged from the hospital on the 10th hospitalization day with the plan of open pulmonary thromboembolectomy in the near future.

   Discussion Top

PE is a relatively rare clinical entity in childhood compared to its frequency in the adult population, with an annual incidence rate of 0.9/100 000 children per year according to the National Hospital Discharge Survey.[2] The incidence curve in childhood depicts a bimodal distribution, with the highest incidence in infants and teenagers.[2],[3] The presence of central venous catheter was reported to be the most common predisposing factor.[3],[4],[5] In another study, however, obesity was found to be the most common risk factor.[6] The following conditions can be listed among other risk factors: immobility, dehydration, malignancy, congenital heart disease, systemic lupus erythematosus, trauma, surgery, prolonged total parenteral nutrition, oral contraceptive pill use, infection, and state of acquired or congenital hypercoagulability/thrombophilia. The patient in our case did not possess any traditional risk factors.

As indicated before, little progress has been achieved in the derivation of clinical decision rules for childhood PE due to its much lower incidence in the child population compared to adult population, confusing presenting complaints, and different risk factors. Despite the presence of some reliable clinical decision rules for the determination of pretest probability of PE in adults such as Wells Criteria[7] and the PE Rule-out Criteria,[8],[9] validated clinical decision rules have not yet been sufficient for the determination of PE in childhood, which may act as a major drawback in the diagnosis of PE in pediatric patients. Moreover, diagnosis in the emergency services may further be compelling due to deceptive clinical presentations which may mimic other common respiratory diseases that are encountered in childhood.

Among the clinical symptoms and signs of PE, pleuritic chest pain, tachycardia, hypoxemia, cough, fever, dyspnea, cyanosis, hypotension, and syncope can be listed. Lack of these symptoms could help to rule out PE. Contrary to the adults among whom dyspnea is the prevailing symptom of PE,[10] the most common symptom in pediatric PE cases is pleuritic chest pain.[11] Relatively less prevalence of chronic pulmonary and cardiac diseases, and thereby, a better preserved cardiopulmonary reserve may explain the lower incidence of dyspnea in childhood PE cases.[12] The presenting symptoms in our case were chest pain for 3 days, dyspnea, and syncope.

As for the diagnosis, there are some laboratory and imaging modalities which guide the physicians. D-dimer and arterial blood gas and ECG are readily available and may be used in the differential diagnosis in suspected patients. Despite the fact that a normal D-dimer level in a patient with lower clinical probability can rule out PE in adults, the same results may not be accountable in pediatric patients due to lack of clinical researches.[7],[13] Hypoxia, hypocapnia, and respiratory alkalosis were proved to be the typical findings in blood gas analysis but might not be observed in all patients.[14] ECG is nonspecific and can reveal sinus tachycardia, right-axis deviation, right bundle brunch deviation, and ST-T segment abnormalities. Although the S1Q3T3 pattern in ECG may suggest the diagnosis of PE, it is neither sensitive nor specific.[15],[16] The S1Q3T3 pattern was present in our case. Ventilation-perfusion lung scanning can be used safely and easily as the initial diagnostic modality even in pediatric patients. A high probability risk scan can readily translate into a definitive diagnosis. Most of the patients, however, fit into low- or intermediate-risk group, which is nondiagnostic.[7]

Factor V Leiden stands for the most commonly encountered genetic inclination to venous thromboembolism, where there appears to be a disrupted anticoagulant response to activated protein C.[17] Prothrombin 20210 gene mutation is also another frequent genetic cause of deep vein thrombosis, which leads to increased blood levels of prothrombin and hence a state of hypercoagulability.[18] Our case, however, was negative for both Factor V Leiden and prothrombin 20210 mutations. MTHFR is an enzyme in homocysteine metabolism, catalyzing the conversion of 5,10-methylenetetrahydrofolate to 5-methyltetrahydrofolate, a cosubstrate for homocysteine remethylation to methionine. Therefore, the defective functioning of this enzyme is likely to lead to accumulation of excessive homocysteine in the bloodstream. Increased circulating homocysteine level was reported to be associated with both arterial and venous thromboembolism.[18] However, studies regarding MTHFR polymorphism (C677T and A1298C) suggested either very weak or no association with venous thromboembolism.[19],[20],[21] As our case was detected to possess heterozygous mutations in MTHFR C677T and MTHFR A1298C, this may in part contribute to the generation of PE in our case. PAI-1 is a serine protease inhibitor which acts as the inhibitor of tissue plasminogen activator and urokinase, the major activators of plasminogen and hence fibrinolysis. Studies regarding the relationship between PAI-1 promoter 4G/5G genotype and deep vein thrombosis revealed conflicting results.[22],[23],[24],[25] A study by Zöller et al. reported that PAI genotype was associated with thrombophilia in individuals with protein S deficiency.[26] Protein S activity was recorded as normal in our case, despite a slight decrease in protein C activity. Protein S and protein C act concurrently as an inhibitor of Factor V and VIII. Protein C deficiency is likely to cause venous thromboembolism.[27],[28] It was reported that adult levels of protein C might not be achieved until the second decade of life.[29] As protein C activity was revealed to be only slightly abated, we are not sure if it might have been implicated in the occurrence of PE in our case.

No matter if the case has been a pediatric patient, any genetic test positivity rendering an individual inclined to the generation of either venous or arterial thrombosis should be assessed in terms of PE in relevant patients which dyspnea, syncope, chest pain, and sudden hypotension or shock in emergency departments. Accordingly, implementation of an emergency echocardiography is very likely to prove helpful in such patients both in the emergency differential diagnosis and administration of therapy. On the other hand, monitoring of serum D-dimer level can be considered in those patients with subtle but relevant clinical symptomology to PE in the differential diagnosis. Moreover, the patients with high serum D-dimer level may be referred for further echocardiographic and even pulmonary CT scan.

Due to controversy and lack of a joint guideline regarding the management of the PE in childhood, the therapy implemented today has mostly been extrapolated from that of adults,[30],[31] which translates into that fact that each medical center should arrange its own multidisciplinary approach. In this regard, the most commonly used therapy for the initial management is either unfractionated heparin or low molecular weight heparin. Furthermore, the patients with findings compatible with massive PE should be treated with pharmacological thrombolytic therapy (recombinant tissue plasminogen activators, urokinase, and streptokinase). Surgical thromboendarterectomy can be spared for the patients with failure of the pharmacological thrombolysis, contraindication to anticoagulation and/or antiaggregation therapy, and high risk of bleeding.[32]

   Conclusion Top

As time goes by, the necessity of reliable and validated clinical decision rules for PE in childhood has been escalating. Although it is relatively easy to diagnose PE in very dramatic cases, patients with subtle symptoms and signs can be challenging in terms of diagnosis, and PE should always be kept in mind as a differential diagnosis in the emergency departments even in pediatric patients with vague but relevant clinical symptomology.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understand that name and initial will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

   References Top

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